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目前国内已利用电脑的多媒体功能,采用临床医学软件作为一种治疗手段,结合个体治疗中的其他方法如改变响度、喉部按摩、半吞咽、改变舌位、减少硬起音、放松、呼吸训练等,达到治疗效果。
[ { "indices": [ 46, 47, 48, 49 ], "label": "pro", "score": 1, "text": "改变响度" }, { "indices": [ 51, 52, 53, 54 ], "label": "pro", "score": 1, "text": "喉部按摩" }, { "indices": [ 56, 57, 58 ], "label": "pro", "score": 1, "text": "半吞咽" }, { "indices": [ 60, 61, 62, 63 ], "label": "pro", "score": 1, "text": "改变舌位" }, { "indices": [ 65, 66, 67, 68, 69 ], "label": "pro", "score": 1, "text": "减少硬起音" }, { "indices": [ 71, 72 ], "label": "pro", "score": 1, "text": "放松" }, { "indices": [ 74, 75, 76, 77 ], "label": "pro", "score": 1, "text": "呼吸训练" } ]
羊水过少妨碍胎儿胸廓的正常活动及肺在子宫内的扩张,造成肺发育不良,生后患儿常有呼吸困难、发绀、呼吸窘迫综合征、气胸及纵隔气肿,可死于呼吸衰竭。
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后尿道瓣膜导致严重尿路梗阻的新生儿可有严重的泌尿系感染、尿毒症、脱水及电解质紊乱。
[ { "indices": [ 0, 1, 2, 3, 4 ], "label": "dis", "score": 1, "text": "后尿道瓣膜" }, { "indices": [ 9, 10, 11, 12 ], "label": "dis", "score": 1, "text": "尿路梗阻" }, { "indices": [ 22, 23, 24, 25, 26 ], "label": "dis", "score": 1, "text": "泌尿系感染" }, { "indices": [ 28, 29, 30 ], "label": "dis", "score": 1, "text": "尿毒症" }, { "indices": [ 32, 33 ], "label": "sym", "score": 1, "text": "脱水" }, { "indices": [ 35, 36, 37, 38, 39 ], "label": "dis", "score": 1, "text": "电解质紊乱" } ]
至婴儿期的患儿可有生长发育迟缓及尿路感染,甚至败血症。
[ { "indices": [ 9, 10, 11, 12, 13, 14 ], "label": "dis", "score": 1, "text": "生长发育迟缓" }, { "indices": [ 16, 17, 18, 19 ], "label": "dis", "score": 1, "text": "尿路感染" }, { "indices": [ 23, 24, 25 ], "label": "dis", "score": 1, "text": "败血症" } ]
学龄期儿童主要表现为排尿时间延长,尿线细,排尿费力,往往有残余尿而导致尿失禁及遗尿。
[ { "indices": [ 10, 11, 12, 13, 14, 15 ], "label": "sym", "score": 1, "text": "排尿时间延长" }, { "indices": [ 11 ], "label": "bod", "score": 1, "text": "尿" }, { "indices": [ 17, 18, 19 ], "label": "sym", "score": 1, "text": "尿线细" }, { "indices": [ 17 ], "label": "bod", "score": 1, "text": "尿" }, { "indices": [ 21, 22, 23, 24 ], "label": "sym", "score": 1, "text": "排尿费力" }, { "indices": [ 22 ], "label": "bod", "score": 1, "text": "尿" }, { "indices": [ 26, 27, 28, 29, 30, 31 ], "label": "sym", "score": 1, "text": "往往有残余尿" }, { "indices": [ 31 ], "label": "bod", "score": 1, "text": "尿" }, { "indices": [ 35, 36, 37 ], "label": "dis", "score": 1, "text": "尿失禁" }, { "indices": [ 39, 40 ], "label": "dis", "score": 1, "text": "遗尿" } ]
VCUG可见前列腺部尿道扩张,其远端尿道极细,膀胱边缘不光滑,有小梁小室形成,可有不同程度的膀胱输尿管反流。
[ { "indices": [ 0, 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13 ], "label": "sym", "score": 1, "text": "VCUG可见前列腺部尿道扩张" }, { "indices": [ 0, 1, 2, 3 ], "label": "pro", "score": 1, "text": "VCUG" }, { "indices": [ 6, 7, 8, 9, 10, 11 ], "label": "bod", "score": 1, "text": "前列腺部尿道" }, { "indices": [ 15, 16, 17, 18, 19, 20, 21 ], "label": "sym", "score": 1, "text": "其远端尿道极细" }, { "indices": [ 16, 17, 18, 19 ], "label": "bod", "score": 1, "text": "远端尿道" }, { "indices": [ 23, 24, 25, 26, 27, 28, 29 ], "label": "sym", "score": 1, "text": "膀胱边缘不光滑" }, { "indices": [ 23, 24, 25, 26 ], "label": "bod", "score": 1, "text": "膀胱边缘" }, { "indices": [ 31, 32, 33, 34, 35, 36, 37 ], "label": "sym", "score": 1, "text": "有小梁小室形成" }, { "indices": [ 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52 ], "label": "sym", "score": 1, "text": "可有不同程度的膀胱输尿管反流" }, { "indices": [ 46, 47, 48, 49, 50 ], "label": "bod", "score": 1, "text": "膀胱输尿管" } ]
后尿道瓣膜的胎儿首先表现为肾积水,但有其特点,常为双侧肾与输尿管积水、膀胱壁增厚及羊水量减少。
[ { "indices": [ 0, 1, 2, 3, 4 ], "label": "dis", "score": 1, "text": "后尿道瓣膜" }, { "indices": [ 13, 14, 15 ], "label": "sym", "score": 1, "text": "肾积水" }, { "indices": [ 13 ], "label": "bod", "score": 1, "text": "肾" }, { "indices": [ 25, 26, 27, 28, 29, 30, 31, 32, 33 ], "label": "sym", "score": 1, "text": "双侧肾与输尿管积水" }, { "indices": [ 25, 26, 27, 28, 29, 30, 31 ], "label": "bod", "score": 1, "text": "双侧肾与输尿管" }, { "indices": [ 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45 ], "label": "sym", "score": 1, "text": "膀胱壁增厚及羊水量减少" }, { "indices": [ 35, 36, 37 ], "label": "bod", "score": 1, "text": "膀胱壁" }, { "indices": [ 41, 42, 43 ], "label": "ite", "score": 1, "text": "羊水量" } ]
对不能经尿道放入内镜的患儿可经膀胱造瘘处放入,顺行电灼瓣膜。
[ { "indices": [ 4, 5 ], "label": "bod", "score": 1, "text": "尿道" }, { "indices": [ 8, 9 ], "label": "equ", "score": 1, "text": "内镜" }, { "indices": [ 15, 16, 17, 18 ], "label": "pro", "score": 1, "text": "膀胱造瘘" }, { "indices": [ 25, 26, 27, 28 ], "label": "pro", "score": 1, "text": "电灼瓣膜" } ]
由于儿童非霍奇金淋巴瘤涉及游走于全身各处的淋巴细胞,其在发病部位和蔓延速度上类似于儿童白血病,倾向于归类为全身性疾病。
[ { "indices": [ 2, 3, 4, 5, 6, 7, 8, 9, 10 ], "label": "dis", "score": 1, "text": "儿童非霍奇金淋巴瘤" }, { "indices": [ 21, 22, 23, 24 ], "label": "bod", "score": 1, "text": "淋巴细胞" }, { "indices": [ 41, 42, 43, 44, 45 ], "label": "dis", "score": 1, "text": "儿童白血病" } ]
儿童NHL治疗的进展是过去20年中最为成功疾病之一。
[ { "indices": [ 0, 1, 2, 3, 4 ], "label": "dis", "score": 1, "text": "儿童NHL" } ]
在美国和发达国家,恶性淋巴瘤(包括NHL及霍奇金病)是继白血病和脑肿瘤之后第三个常见的儿童恶性肿瘤,在小于20岁的少儿中占儿童恶性肿瘤的15%。
[ { "indices": [ 9, 10, 11, 12, 13 ], "label": "dis", "score": 1, "text": "恶性淋巴瘤" }, { "indices": [ 17, 18, 19 ], "label": "dis", "score": 1, "text": "NHL" }, { "indices": [ 21, 22, 23, 24 ], "label": "dis", "score": 1, "text": "霍奇金病" }, { "indices": [ 28, 29, 30 ], "label": "dis", "score": 1, "text": "白血病" }, { "indices": [ 32, 33, 34 ], "label": "dis", "score": 1, "text": "脑肿瘤" }, { "indices": [ 43, 44, 45, 46, 47, 48 ], "label": "dis", "score": 1, "text": "儿童恶性肿瘤" }, { "indices": [ 61, 62, 63, 64, 65, 66 ], "label": "dis", "score": 1, "text": "儿童恶性肿瘤" } ]
15~19岁之间的儿童霍奇金病发病率几乎是NHL的两倍。
[ { "indices": [ 9, 10, 11, 12, 13, 14 ], "label": "dis", "score": 1, "text": "儿童霍奇金病" }, { "indices": [ 21, 22, 23 ], "label": "dis", "score": 1, "text": "NHL" } ]
NHL发病率因年龄有显著差异;5岁以内儿童NHL并不常见,仅占所有肿瘤的3%,NHL的发病率在整个人生中持续上升,在10岁以上的儿童肿瘤中占8%~9%。
[ { "indices": [ 0, 1, 2 ], "label": "dis", "score": 1, "text": "NHL" }, { "indices": [ 21, 22, 23 ], "label": "dis", "score": 1, "text": "NHL" }, { "indices": [ 33, 34 ], "label": "dis", "score": 1, "text": "肿瘤" }, { "indices": [ 39, 40, 41 ], "label": "dis", "score": 1, "text": "NHL" }, { "indices": [ 66, 67 ], "label": "dis", "score": 1, "text": "肿瘤" } ]
自从对正常免疫系统有了更深的认识,免疫诊断技术也应用于NHL的诊断,NHL是淋巴细胞在分化成熟过程中某个时期细胞恶变的结果。
[ { "indices": [ 5, 6, 7, 8 ], "label": "bod", "score": 1, "text": "免疫系统" }, { "indices": [ 17, 18, 19, 20 ], "label": "pro", "score": 1, "text": "免疫诊断" }, { "indices": [ 27, 28, 29 ], "label": "dis", "score": 1, "text": "NHL" }, { "indices": [ 34, 35, 36 ], "label": "dis", "score": 1, "text": "NHL" }, { "indices": [ 38, 39, 40, 41 ], "label": "bod", "score": 1, "text": "淋巴细胞" }, { "indices": [ 54, 55, 56, 57 ], "label": "sym", "score": 1, "text": "细胞恶变" }, { "indices": [ 54, 55 ], "label": "bod", "score": 1, "text": "细胞" } ]
大约40%~50%儿童NHL都起自T细胞系;同等比例的病例则来自表达表面免疫球蛋白的B细胞肿瘤。
[ { "indices": [ 9, 10, 11, 12, 13 ], "label": "dis", "score": 1, "text": "儿童NHL" }, { "indices": [ 17, 18, 19, 20 ], "label": "bod", "score": 1, "text": "T细胞系" }, { "indices": [ 34, 35, 36, 37, 38, 39, 40 ], "label": "bod", "score": 1, "text": "表面免疫球蛋白" }, { "indices": [ 42, 43, 44, 45, 46 ], "label": "dis", "score": 1, "text": "B细胞肿瘤" } ]
仅有不到10%的儿童NHL为缺乏表面免疫球蛋白的早前B细胞肿瘤。
[ { "indices": [ 8, 9, 10, 11, 12 ], "label": "dis", "score": 1, "text": "儿童NHL" }, { "indices": [ 16, 17, 18, 19, 20, 21, 22 ], "label": "bod", "score": 1, "text": "表面免疫球蛋白" }, { "indices": [ 26, 27, 28, 29, 30 ], "label": "dis", "score": 1, "text": "B细胞肿瘤" } ]
NHL的现代分类方案包含了淋巴细胞性白血病,从而确认了NHL与ALL之间的紧密关系,并认识到从骨髓浸润的程度区别ALL和NHL并不合理。
[ { "indices": [ 0, 1, 2 ], "label": "dis", "score": 1, "text": "NHL" }, { "indices": [ 13, 14, 15, 16, 17, 18, 19, 20 ], "label": "dis", "score": 1, "text": "淋巴细胞性白血病" }, { "indices": [ 27, 28, 29 ], "label": "dis", "score": 1, "text": "NHL" }, { "indices": [ 31, 32, 33 ], "label": "dis", "score": 1, "text": "ALL" }, { "indices": [ 47, 48 ], "label": "bod", "score": 1, "text": "骨髓" }, { "indices": [ 47, 48, 49, 50 ], "label": "sym", "score": 1, "text": "骨髓浸润" }, { "indices": [ 56, 57, 58 ], "label": "dis", "score": 1, "text": "ALL" }, { "indices": [ 60, 61, 62 ], "label": "dis", "score": 1, "text": "NHL" } ]
增殖抗原Ki-67高表达。
[ { "indices": [ 0, 1, 2, 3, 4, 5, 6, 7, 8 ], "label": "bod", "score": 1, "text": "增殖抗原Ki-67" }, { "indices": [ 0, 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11 ], "label": "sym", "score": 1, "text": "增殖抗原Ki-67高表达" } ]
绝大多数Burkitt淋巴瘤存在非随机染色体易位[t(8;14)(q24;q32)],结果是8号染色体上的MYC原癌基因与位于14号染色体的免疫球蛋白重链基因融合。
[ { "indices": [ 4, 5, 6, 7, 8, 9, 10, 11, 12, 13 ], "label": "dis", "score": 1, "text": "Burkitt淋巴瘤" }, { "indices": [ 16, 17, 18, 19, 20, 21, 22, 23 ], "label": "sym", "score": 1, "text": "非随机染色体易位" }, { "indices": [ 19, 20, 21 ], "label": "bod", "score": 1, "text": "染色体" }, { "indices": [ 46, 47, 48, 49, 50 ], "label": "bod", "score": 1, "text": "8号染色体" }, { "indices": [ 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76, 77, 78, 79, 80 ], "label": "sym", "score": 1, "text": "8号染色体上的MYC原癌基因与位于14号染色体的免疫球蛋白重链基因融合" }, { "indices": [ 53, 54, 55, 56, 57, 58, 59 ], "label": "bod", "score": 1, "text": "MYC原癌基因" }, { "indices": [ 63, 64, 65, 66, 67, 68 ], "label": "bod", "score": 1, "text": "14号染色体" }, { "indices": [ 70, 71, 72, 73, 74, 75, 76, 77, 78 ], "label": "bod", "score": 1, "text": "免疫球蛋白重链基因" } ]
ALCL约占儿童NHL的8%~12%,或儿童大细胞淋巴瘤的30%~40%。
[ { "indices": [ 0, 1, 2, 3 ], "label": "dis", "score": 1, "text": "ALCL" }, { "indices": [ 6, 7, 8, 9, 10 ], "label": "dis", "score": 1, "text": "儿童NHL" }, { "indices": [ 20, 21, 22, 23, 24, 25, 26, 27 ], "label": "dis", "score": 1, "text": "儿童大细胞淋巴瘤" } ]
同一系列(T或B)的白血病或淋巴瘤在病理/细胞形态学、免疫学、生物遗传学均无法鉴别白血病还是淋巴瘤,但临床上前者骨髓原发,而后者骨髓外局部原发。
[ { "indices": [ 10, 11, 12 ], "label": "dis", "score": 1, "text": "白血病" }, { "indices": [ 14, 15, 16 ], "label": "dis", "score": 1, "text": "淋巴瘤" }, { "indices": [ 41, 42, 43 ], "label": "dis", "score": 1, "text": "白血病" }, { "indices": [ 46, 47, 48 ], "label": "dis", "score": 1, "text": "淋巴瘤" }, { "indices": [ 56, 57 ], "label": "bod", "score": 1, "text": "骨髓" }, { "indices": [ 56, 57, 58, 59 ], "label": "sym", "score": 1, "text": "骨髓原发" }, { "indices": [ 64, 65, 66 ], "label": "bod", "score": 1, "text": "骨髓外" }, { "indices": [ 64, 65, 66, 67, 68, 69, 70 ], "label": "sym", "score": 1, "text": "骨髓外局部原发" } ]
有时可表现为肠套叠、胃肠道出血、阑尾炎样表现、甚至少数病人发生肠穿孔等急腹症。
[ { "indices": [ 6, 7, 8 ], "label": "dis", "score": 1, "text": "肠套叠" }, { "indices": [ 10, 11, 12, 13, 14 ], "label": "dis", "score": 1, "text": "胃肠道出血" }, { "indices": [ 16, 17, 18 ], "label": "dis", "score": 1, "text": "阑尾炎" }, { "indices": [ 31, 32, 33 ], "label": "dis", "score": 1, "text": "肠穿孔" }, { "indices": [ 35, 36, 37 ], "label": "dis", "score": 1, "text": "急腹症" } ]
右下腹肿块较多见,需与炎性阑尾包块、阑尾炎鉴别。
[ { "indices": [ 0, 1, 2, 3, 4 ], "label": "sym", "score": 1, "text": "右下腹肿块" }, { "indices": [ 0, 1, 2 ], "label": "bod", "score": 1, "text": "右下腹" }, { "indices": [ 11, 12, 13, 14, 15, 16 ], "label": "dis", "score": 1, "text": "炎性阑尾包块" }, { "indices": [ 18, 19, 20 ], "label": "dis", "score": 1, "text": "阑尾炎" } ]
鼻咽部也是较多见的原发部位,可表现为鼻塞、打鼾、血性分泌物及吸气性呼吸困难。
[ { "indices": [ 0, 1, 2 ], "label": "bod", "score": 1, "text": "鼻咽部" }, { "indices": [ 18, 19 ], "label": "sym", "score": 1, "text": "鼻塞" }, { "indices": [ 18 ], "label": "bod", "score": 1, "text": "鼻" }, { "indices": [ 21, 22 ], "label": "sym", "score": 1, "text": "打鼾" }, { "indices": [ 24, 25, 26, 27, 28 ], "label": "sym", "score": 1, "text": "血性分泌物" }, { "indices": [ 26, 27, 28 ], "label": "bod", "score": 1, "text": "分泌物" }, { "indices": [ 30, 31, 32, 33, 34, 35, 36 ], "label": "sym", "score": 1, "text": "吸气性呼吸困难" } ]
病理(细胞)形态学满足NHL的基本诊断,免疫学已成为当今NHL诊断分型的必要手段,有条件时应尽可能进行相关亚型的分子生物学特征检测,如伯基特淋巴瘤常存在t(8;14)及其变异,而间变大细胞淋巴瘤常存在t(2;5)及其变异,使诊断更为可靠。
[ { "indices": [ 11, 12, 13 ], "label": "dis", "score": 1, "text": "NHL" }, { "indices": [ 28, 29, 30 ], "label": "dis", "score": 1, "text": "NHL" }, { "indices": [ 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64 ], "label": "pro", "score": 1, "text": "亚型的分子生物学特征检测" }, { "indices": [ 67, 68, 69, 70, 71, 72 ], "label": "dis", "score": 1, "text": "伯基特淋巴瘤" }, { "indices": [ 74, 75, 76, 77, 78, 79, 80, 81, 82, 83, 84, 85, 86 ], "label": "sym", "score": 1, "text": "存在t(8;14)及其变异" }, { "indices": [ 76, 77, 78, 79, 80, 81, 82 ], "label": "bod", "score": 1, "text": "t(8;14)" }, { "indices": [ 89, 90, 91, 92, 93, 94, 95, 96 ], "label": "dis", "score": 1, "text": "间变大细胞淋巴瘤" }, { "indices": [ 98, 99, 100, 101, 102, 103, 104, 105, 106, 107, 108, 109 ], "label": "sym", "score": 1, "text": "存在t(2;5)及其变异" }, { "indices": [ 100, 101, 102, 103, 104, 105 ], "label": "bod", "score": 1, "text": "t(2;5)" } ]
有胸膜腔积液或心包积液时可引流改善症状。
[ { "indices": [ 1, 2, 3, 4, 5 ], "label": "sym", "score": 1, "text": "胸膜腔积液" }, { "indices": [ 1, 2, 3 ], "label": "bod", "score": 1, "text": "胸膜腔" }, { "indices": [ 7, 8, 9, 10 ], "label": "sym", "score": 1, "text": "心包积液" }, { "indices": [ 7, 8 ], "label": "bod", "score": 1, "text": "心包" }, { "indices": [ 13, 14 ], "label": "pro", "score": 1, "text": "引流" } ]
刚开始治疗时,输入液体多时可致原有的胸腹腔积液增多,必要时可留置引流。
[ { "indices": [ 18, 19, 20, 21, 22 ], "label": "bod", "score": 1, "text": "胸腹腔积液" }, { "indices": [ 18, 19, 20, 21, 22, 23, 24 ], "label": "sym", "score": 1, "text": "胸腹腔积液增多" }, { "indices": [ 30, 31, 32, 33 ], "label": "pro", "score": 1, "text": "留置引流" } ]
与HD有关的其他染色体异常还有:常染色体2、10和13缺失症,11、22部分三体综合征等。
[ { "indices": [ 1, 2 ], "label": "dis", "score": 1, "text": "HD" }, { "indices": [ 8, 9, 10 ], "label": "bod", "score": 1, "text": "染色体" }, { "indices": [ 8, 9, 10, 11, 12 ], "label": "sym", "score": 1, "text": "染色体异常" }, { "indices": [ 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29 ], "label": "dis", "score": 1, "text": "常染色体2、10和13缺失症" }, { "indices": [ 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42 ], "label": "dis", "score": 1, "text": "11、22部分三体综合征" } ]
同时伴发HD、小头畸形、精神发育延滞及面部异常(眼距过宽、球形角膜、眉毛过浓及耳前倾等),是一类与EDNRB基因305位点色氨酸突变有关的综合征;伴发HD的先天性通气不足综合征(原发性肺泡换气不足,Haddad综合征)可能与GDVF和RET基因突变的不完全外显率相关,文献报道161例原发性肺泡换气不足综合征患儿有27%伴发HD;Ⅱ型多发性内分泌瘤(MEN-Ⅱ)同时发生甲状腺髓样癌、嗜铬细胞瘤及多发性黏液瘤,偶有伴发HD。
[ { "indices": [ 4, 5 ], "label": "dis", "score": 1, "text": "HD" }, { "indices": [ 7, 8, 9, 10 ], "label": "dis", "score": 1, "text": "小头畸形" }, { "indices": [ 12, 13, 14, 15, 16, 17 ], "label": "sym", "score": 1, "text": "精神发育延滞" }, { "indices": [ 19, 20, 21, 22 ], "label": "sym", "score": 1, "text": "面部异常" }, { "indices": [ 19, 20 ], "label": "bod", "score": 1, "text": "面部" }, { "indices": [ 24, 25, 26, 27 ], "label": "sym", "score": 1, "text": "眼距过宽" }, { "indices": [ 24, 25 ], "label": "bod", "score": 1, "text": "眼距" }, { "indices": [ 29, 30, 31, 32 ], "label": "dis", "score": 1, "text": "球形角膜" }, { "indices": [ 34, 35, 36, 37 ], "label": "sym", "score": 1, "text": "眉毛过浓" }, { "indices": [ 34, 35 ], "label": "bod", "score": 1, "text": "眉毛" }, { "indices": [ 39, 40, 41 ], "label": "sym", "score": 1, "text": "耳前倾" }, { "indices": [ 39 ], "label": "bod", "score": 1, "text": "耳" }, { "indices": [ 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71 ], "label": "dis", "score": 1, "text": "与EDNRB基因305位点色氨酸突变有关的综合征" }, { "indices": [ 75, 76 ], "label": "dis", "score": 1, "text": "HD" }, { "indices": [ 78, 79, 80, 81, 82, 83, 84, 85, 86, 87 ], "label": "dis", "score": 1, "text": "先天性通气不足综合征" }, { "indices": [ 89, 90, 91, 92, 93, 94, 95, 96, 97 ], "label": "dis", "score": 1, "text": "原发性肺泡换气不足" }, { "indices": [ 99, 100, 101, 102, 103, 104, 105, 106, 107 ], "label": "dis", "score": 1, "text": "Haddad综合征" }, { "indices": [ 112, 113, 114, 115 ], "label": "bod", "score": 1, "text": "GDVF" }, { "indices": [ 117, 118, 119, 120, 121 ], "label": "bod", "score": 1, "text": "RET基因" }, { "indices": [ 117, 118, 119, 120, 121, 122, 123 ], "label": "sym", "score": 1, "text": "RET基因突变" }, { "indices": [ 142, 143, 144, 145, 146, 147, 148, 149, 150, 151, 152, 153 ], "label": "dis", "score": 1, "text": "原发性肺泡换气不足综合征" }, { "indices": [ 162, 163 ], "label": "dis", "score": 1, "text": "HD" }, { "indices": [ 165, 166, 167, 168, 169, 170, 171, 172, 173 ], "label": "dis", "score": 1, "text": "Ⅱ型多发性内分泌瘤" }, { "indices": [ 175, 176, 177, 178, 179 ], "label": "dis", "score": 1, "text": "MEN-Ⅱ" }, { "indices": [ 185, 186, 187, 188, 189, 190 ], "label": "dis", "score": 1, "text": "甲状腺髓样癌" }, { "indices": [ 192, 193, 194, 195, 196 ], "label": "dis", "score": 1, "text": "嗜铬细胞瘤" }, { "indices": [ 198, 199, 200, 201, 202, 203 ], "label": "dis", "score": 1, "text": "多发性黏液瘤" }, { "indices": [ 209, 210 ], "label": "dis", "score": 1, "text": "HD" } ]
大多数HD患儿在新生儿期即出现肠梗阻或严重便秘,主要表现为生后24小时内不能排出胎粪、腹胀和呕吐,某些婴儿甚至可出现完全性肠梗阻;也有患儿在生后几周至几个月内几乎没有症状,以后才出现顽固性便秘,尤其在调整饮食结构,如停母乳改为牛乳或添加固体食物时,便秘加重。
[ { "indices": [ 3, 4 ], "label": "dis", "score": 1, "text": "HD" }, { "indices": [ 15, 16, 17 ], "label": "dis", "score": 1, "text": "肠梗阻" }, { "indices": [ 19, 20, 21, 22 ], "label": "sym", "score": 1, "text": "严重便秘" }, { "indices": [ 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41 ], "label": "sym", "score": 1, "text": "24小时内不能排出胎粪" }, { "indices": [ 41 ], "label": "bod", "score": 1, "text": "粪" }, { "indices": [ 43, 44 ], "label": "sym", "score": 1, "text": "腹胀" }, { "indices": [ 43 ], "label": "bod", "score": 1, "text": "腹" }, { "indices": [ 46, 47 ], "label": "sym", "score": 1, "text": "呕吐" }, { "indices": [ 58, 59, 60, 61, 62, 63 ], "label": "dis", "score": 1, "text": "完全性肠梗阻" }, { "indices": [ 91, 92, 93, 94, 95 ], "label": "sym", "score": 1, "text": "顽固性便秘" }, { "indices": [ 124, 125, 126, 127 ], "label": "sym", "score": 1, "text": "便秘加重" } ]
那些确诊较晚的患儿,往往因长期便秘,导致严重腹胀,出现蛙状腹,触诊可及多个粪块,且多见小肠结肠炎。
[ { "indices": [ 13, 14, 15, 16 ], "label": "sym", "score": 1, "text": "长期便秘" }, { "indices": [ 20, 21, 22, 23 ], "label": "sym", "score": 1, "text": "严重腹胀" }, { "indices": [ 22 ], "label": "bod", "score": 1, "text": "腹" }, { "indices": [ 25, 26, 27, 28, 29 ], "label": "sym", "score": 1, "text": "出现蛙状腹" }, { "indices": [ 29 ], "label": "bod", "score": 1, "text": "腹" }, { "indices": [ 31, 32 ], "label": "pro", "score": 1, "text": "触诊" }, { "indices": [ 35, 36, 37, 38 ], "label": "sym", "score": 1, "text": "多个粪块" }, { "indices": [ 37, 38 ], "label": "bod", "score": 1, "text": "粪块" }, { "indices": [ 43, 44, 45, 46, 47 ], "label": "dis", "score": 1, "text": "小肠结肠炎" } ]
小肠结肠炎是最严重的巨结肠并发症,可致全身性中毒,危及生命。
[ { "indices": [ 0, 1, 2, 3, 4 ], "label": "dis", "score": 1, "text": "小肠结肠炎" }, { "indices": [ 10, 11, 12, 13, 14, 15 ], "label": "dis", "score": 1, "text": "巨结肠并发症" }, { "indices": [ 17, 18, 19, 20, 21, 22, 23 ], "label": "sym", "score": 1, "text": "可致全身性中毒" } ]
临床表现以发热、胆汁样呕吐、喷射样腹泻、腹胀、脱水和休克为特点。
[ { "indices": [ 5, 6 ], "label": "sym", "score": 1, "text": "发热" }, { "indices": [ 8, 9, 10, 11, 12 ], "label": "sym", "score": 1, "text": "胆汁样呕吐" }, { "indices": [ 8, 9 ], "label": "bod", "score": 1, "text": "胆汁" }, { "indices": [ 14, 15, 16, 17, 18 ], "label": "sym", "score": 1, "text": "喷射样腹泻" }, { "indices": [ 17 ], "label": "bod", "score": 1, "text": "腹" }, { "indices": [ 20, 21 ], "label": "sym", "score": 1, "text": "腹胀" }, { "indices": [ 20 ], "label": "bod", "score": 1, "text": "腹" }, { "indices": [ 23, 24 ], "label": "sym", "score": 1, "text": "脱水" }, { "indices": [ 26, 27 ], "label": "dis", "score": 1, "text": "休克" } ]
节细胞缺乏段近端肠管黏液溃疡和缺血性坏死可致败血症、肠腔积气症和肠穿孔,有报告自发性穿孔率为3%,无神经节细胞肠段长度与穿孔发生率密切相关。
[ { "indices": [ 0, 1, 2, 3, 4 ], "label": "sym", "score": 1, "text": "节细胞缺乏" }, { "indices": [ 0, 1, 2 ], "label": "bod", "score": 1, "text": "节细胞" }, { "indices": [ 5, 6, 7, 8, 9, 10, 11, 12, 13 ], "label": "dis", "score": 1, "text": "段近端肠管黏液溃疡" }, { "indices": [ 15, 16, 17, 18, 19 ], "label": "dis", "score": 1, "text": "缺血性坏死" }, { "indices": [ 22, 23, 24 ], "label": "dis", "score": 1, "text": "败血症" }, { "indices": [ 26, 27, 28, 29, 30 ], "label": "dis", "score": 1, "text": "肠腔积气症" }, { "indices": [ 32, 33, 34 ], "label": "dis", "score": 1, "text": "肠穿孔" }, { "indices": [ 49, 50, 51, 52, 53, 54, 55, 56 ], "label": "bod", "score": 1, "text": "无神经节细胞肠段" } ]
肛指检查可发现肛门括约肌痉挛与直肠壶腹部空虚感。
[ { "indices": [ 0, 1, 2, 3 ], "label": "pro", "score": 1, "text": "肛指检查" }, { "indices": [ 7, 8, 9, 10, 11, 12, 13 ], "label": "sym", "score": 1, "text": "肛门括约肌痉挛" }, { "indices": [ 7, 8, 9, 10, 11 ], "label": "bod", "score": 1, "text": "肛门括约肌" }, { "indices": [ 15, 16, 17, 18, 19, 20, 21, 22 ], "label": "sym", "score": 1, "text": "直肠壶腹部空虚感" }, { "indices": [ 15, 16, 17, 18, 19 ], "label": "bod", "score": 1, "text": "直肠壶腹部" } ]
肛指检查、插入肛管或体温计、或清洁灌肠时出现粪汁及大量气体喷出,应高度怀疑小肠结肠炎。
[ { "indices": [ 0, 1, 2, 3 ], "label": "pro", "score": 1, "text": "肛指检查" }, { "indices": [ 7, 8 ], "label": "equ", "score": 1, "text": "肛管" }, { "indices": [ 10, 11, 12 ], "label": "equ", "score": 1, "text": "体温计" }, { "indices": [ 15, 16, 17, 18 ], "label": "pro", "score": 1, "text": "清洁灌肠" }, { "indices": [ 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30 ], "label": "sym", "score": 1, "text": "出现粪汁及大量气体喷出" }, { "indices": [ 22, 23 ], "label": "bod", "score": 1, "text": "粪汁" }, { "indices": [ 37, 38, 39, 40, 41 ], "label": "dis", "score": 1, "text": "小肠结肠炎" } ]
通常排便排气后,短时间内症状缓解,但不久又再次出现腹胀。
[ { "indices": [ 3 ], "label": "bod", "score": 1, "text": "便" }, { "indices": [ 8, 9, 10, 11, 12, 13, 14, 15 ], "label": "sym", "score": 1, "text": "短时间内症状缓解" }, { "indices": [ 18, 19, 20, 21, 22, 23, 24, 25, 26 ], "label": "sym", "score": 1, "text": "不久又再次出现腹胀" }, { "indices": [ 25 ], "label": "bod", "score": 1, "text": "腹" } ]
确诊HD手段包括影像学检查、直肠肛管测压和直肠黏液活检组化分析等。
[ { "indices": [ 2, 3 ], "label": "dis", "score": 1, "text": "HD" }, { "indices": [ 8, 9, 10, 11, 12 ], "label": "pro", "score": 1, "text": "影像学检查" }, { "indices": [ 14, 15, 16, 17, 18, 19 ], "label": "pro", "score": 1, "text": "直肠肛管测压" }, { "indices": [ 21, 22, 23, 24, 25, 26, 27, 28, 29, 30 ], "label": "pro", "score": 1, "text": "直肠黏液活检组化分析" } ]
另外,新生儿败血症和脑损伤也可致胎粪排出延迟。
[ { "indices": [ 3, 4, 5, 6, 7, 8 ], "label": "dis", "score": 1, "text": "新生儿败血症" }, { "indices": [ 10, 11, 12 ], "label": "dis", "score": 1, "text": "脑损伤" }, { "indices": [ 16, 17, 18, 19, 20, 21 ], "label": "sym", "score": 1, "text": "胎粪排出延迟" }, { "indices": [ 17 ], "label": "bod", "score": 1, "text": "粪" } ]
但是,如果发现新生儿或婴儿存在肠梗阻或小肠结肠炎症状,应首先怀疑HD及相关ENS疾病,行钡剂灌肠,直肠吸引活检以及直肠测压等检查,以明确诊断。
[ { "indices": [ 15, 16, 17 ], "label": "dis", "score": 1, "text": "肠梗阻" }, { "indices": [ 19, 20, 21, 22, 23 ], "label": "dis", "score": 1, "text": "小肠结肠炎" }, { "indices": [ 32, 33 ], "label": "dis", "score": 1, "text": "HD" }, { "indices": [ 37, 38, 39, 40, 41 ], "label": "dis", "score": 1, "text": "ENS疾病" }, { "indices": [ 43, 44, 45, 46, 47 ], "label": "pro", "score": 1, "text": "行钡剂灌肠" }, { "indices": [ 49, 50, 51, 52, 53, 54 ], "label": "pro", "score": 1, "text": "直肠吸引活检" }, { "indices": [ 57, 58, 59, 60 ], "label": "pro", "score": 1, "text": "直肠测压" } ]
饮食习惯不当、心理障碍、药物性肠蠕动不良、代谢或内分泌疾病(如尿毒症或甲状腺功能减低)等均可能引起慢性便秘与肠梗阻;其他可致便秘的因素包括内源性肠神经功能疾病(糖尿病或家族性自主神经功能异常)、中枢神经系统病变和平滑肌功能失调等;收缩蛋白异常和结缔组织病(如硬皮病或皮肌炎)同样可致持久性便秘。
[ { "indices": [ 7, 8, 9, 10 ], "label": "sym", "score": 1, "text": "心理障碍" }, { "indices": [ 12, 13, 14, 15, 16, 17, 18, 19 ], "label": "sym", "score": 1, "text": "药物性肠蠕动不良" }, { "indices": [ 15 ], "label": "bod", "score": 1, "text": "肠" }, { "indices": [ 21, 22, 23, 24, 25, 26, 27, 28 ], "label": "dis", "score": 1, "text": "代谢或内分泌疾病" }, { "indices": [ 31, 32, 33 ], "label": "dis", "score": 1, "text": "尿毒症" }, { "indices": [ 35, 36, 37, 38, 39, 40, 41 ], "label": "dis", "score": 1, "text": "甲状腺功能减低" }, { "indices": [ 49, 50, 51, 52 ], "label": "dis", "score": 1, "text": "慢性便秘" }, { "indices": [ 54, 55, 56 ], "label": "dis", "score": 1, "text": "肠梗阻" }, { "indices": [ 69, 70, 71, 72, 73, 74, 75, 76, 77, 78 ], "label": "dis", "score": 1, "text": "内源性肠神经功能疾病" }, { "indices": [ 80, 81, 82 ], "label": "dis", "score": 1, "text": "糖尿病" }, { "indices": [ 84, 85, 86, 87, 88, 89, 90, 91, 92, 93, 94 ], "label": "dis", "score": 1, "text": "家族性自主神经功能异常" }, { "indices": [ 97, 98, 99, 100, 101, 102, 103, 104 ], "label": "dis", "score": 1, "text": "中枢神经系统病变" }, { "indices": [ 106, 107, 108, 109, 110, 111, 112 ], "label": "dis", "score": 1, "text": "平滑肌功能失调" }, { "indices": [ 115, 116, 117, 118, 119, 120 ], "label": "dis", "score": 1, "text": "收缩蛋白异常" }, { "indices": [ 122, 123, 124, 125, 126 ], "label": "dis", "score": 1, "text": "结缔组织病" }, { "indices": [ 129, 130, 131 ], "label": "dis", "score": 1, "text": "硬皮病" }, { "indices": [ 133, 134, 135 ], "label": "dis", "score": 1, "text": "皮肌炎" }, { "indices": [ 141, 142, 143, 144, 145 ], "label": "sym", "score": 1, "text": "持久性便秘" } ]
钡剂造影可发现小结肠,超声或静脉尿路造影可发现肾盂积水和巨膀胱,以此与HD鉴别。
[ { "indices": [ 0, 1, 2, 3 ], "label": "pro", "score": 1, "text": "钡剂造影" }, { "indices": [ 7, 8, 9 ], "label": "dis", "score": 1, "text": "小结肠" }, { "indices": [ 11, 12 ], "label": "pro", "score": 1, "text": "超声" }, { "indices": [ 14, 15, 16, 17, 18, 19 ], "label": "pro", "score": 1, "text": "静脉尿路造影" }, { "indices": [ 23, 24, 25, 26 ], "label": "dis", "score": 1, "text": "肾盂积水" }, { "indices": [ 28, 29, 30 ], "label": "dis", "score": 1, "text": "巨膀胱" }, { "indices": [ 35, 36 ], "label": "dis", "score": 1, "text": "HD" } ]
外周血的异常变化不能作为白血病的诊断依据,当临床怀疑白血病时,需及时作骨髓穿刺涂片以明确诊断。
[ { "indices": [ 0, 1, 2, 3, 4, 5, 6, 7 ], "label": "sym", "score": 1, "text": "外周血的异常变化" }, { "indices": [ 0, 1, 2 ], "label": "bod", "score": 1, "text": "外周血" }, { "indices": [ 12, 13, 14 ], "label": "dis", "score": 1, "text": "白血病" }, { "indices": [ 26, 27, 28 ], "label": "dis", "score": 1, "text": "白血病" }, { "indices": [ 35, 36, 37, 38, 39, 40 ], "label": "pro", "score": 1, "text": "骨髓穿刺涂片" } ]
绝大多数白血病骨髓涂片表现为有核细胞增生活跃、明显活跃或极度活跃,5%~10%的急性白血病骨髓增生低下,称之为低增生性白血病。
[ { "indices": [ 4, 5, 6 ], "label": "dis", "score": 1, "text": "白血病" }, { "indices": [ 7, 8, 9, 10 ], "label": "ite", "score": 1, "text": "骨髓涂片" }, { "indices": [ 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31 ], "label": "sym", "score": 1, "text": "有核细胞增生活跃、明显活跃或极度活跃" }, { "indices": [ 14, 15, 16, 17 ], "label": "bod", "score": 1, "text": "有核细胞" }, { "indices": [ 40, 41, 42, 43, 44 ], "label": "dis", "score": 1, "text": "急性白血病" }, { "indices": [ 45, 46, 47, 48, 49, 50 ], "label": "dis", "score": 1, "text": "骨髓增生低下" }, { "indices": [ 55, 56, 57, 58, 59, 60, 61 ], "label": "dis", "score": 1, "text": "低增生性白血病" } ]
诊断ALL的主要依据是骨髓有核细胞中原始和幼稚淋巴细胞总和>30%,此时正常的红系、巨核细胞系、粒系常明显受抑甚至消失。
[ { "indices": [ 2, 3, 4 ], "label": "dis", "score": 1, "text": "ALL" }, { "indices": [ 11, 12, 13, 14, 15, 16 ], "label": "bod", "score": 1, "text": "骨髓有核细胞" }, { "indices": [ 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32 ], "label": "sym", "score": 1, "text": "骨髓有核细胞中原始和幼稚淋巴细胞总和>30%" }, { "indices": [ 18, 19, 20, 21, 22, 23, 24, 25, 26 ], "label": "bod", "score": 1, "text": "原始和幼稚淋巴细胞" }, { "indices": [ 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58 ], "label": "sym", "score": 1, "text": "正常的红系、巨核细胞系、粒系常明显受抑甚至消失" }, { "indices": [ 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49 ], "label": "bod", "score": 1, "text": "红系、巨核细胞系、粒系" } ]
染色体>46条时称为超二倍体,而>50条的超二倍体者预后较好。
[ { "indices": [ 0, 1, 2 ], "label": "bod", "score": 1, "text": "染色体" } ]
推荐用大剂量甲氨蝶呤(HDMTX)和鞘内化疗进行CNSL预防。
[ { "indices": [ 6, 7, 8, 9 ], "label": "dru", "score": 1, "text": "甲氨蝶呤" }, { "indices": [ 11, 12, 13, 14, 15 ], "label": "dru", "score": 1, "text": "HDMTX" }, { "indices": [ 18, 19, 20, 21 ], "label": "pro", "score": 1, "text": "鞘内化疗" }, { "indices": [ 24, 25, 26, 27 ], "label": "dis", "score": 1, "text": "CNSL" } ]
(2)预防感染:注意食品及环境卫生,减少感染机会。
[ { "indices": [ 5, 6 ], "label": "dis", "score": 1, "text": "感染" }, { "indices": [ 20, 21 ], "label": "dis", "score": 1, "text": "感染" } ]
(3)应用L-ASP时宜低脂饮食,减少合并急性胰腺炎的机会;注意血白蛋白水平,明显低下时及时补充,以避免低蛋白血症加重感染。
[ { "indices": [ 5, 6, 7, 8, 9 ], "label": "dru", "score": 1, "text": "L-ASP" }, { "indices": [ 12, 13, 14, 15 ], "label": "pro", "score": 1, "text": "低脂饮食" }, { "indices": [ 21, 22, 23, 24, 25 ], "label": "dis", "score": 1, "text": "急性胰腺炎" }, { "indices": [ 32, 33, 34, 35 ], "label": "bod", "score": 1, "text": "血白蛋白" }, { "indices": [ 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42 ], "label": "sym", "score": 1, "text": "血白蛋白水平,明显低下" }, { "indices": [ 52, 53, 54, 55, 56 ], "label": "dis", "score": 1, "text": "低蛋白血症" }, { "indices": [ 59, 60 ], "label": "dis", "score": 1, "text": "感染" } ]
自由基以及抗氧化剂缺乏在慢性胰腺炎的形成和发展中都起了很重要的作用。
[ { "indices": [ 0, 1, 2, 3, 4, 5, 6, 7, 8, 9, 10 ], "label": "sym", "score": 1, "text": "自由基以及抗氧化剂缺乏" }, { "indices": [ 12, 13, 14, 15, 16 ], "label": "dis", "score": 1, "text": "慢性胰腺炎" } ]
在钙化性胰腺炎,随着胰腺的钙化,疼痛减轻,但随之出现内、外分泌功能不足。
[ { "indices": [ 1, 2, 3, 4, 5, 6 ], "label": "dis", "score": 1, "text": "钙化性胰腺炎" }, { "indices": [ 10, 11 ], "label": "bod", "score": 1, "text": "胰腺" }, { "indices": [ 10, 11, 12, 13, 14 ], "label": "sym", "score": 1, "text": "胰腺的钙化" }, { "indices": [ 16, 17, 18, 19 ], "label": "sym", "score": 1, "text": "疼痛减轻" }, { "indices": [ 26, 27, 28, 29, 30, 31, 32, 33, 34 ], "label": "sym", "score": 1, "text": "内、外分泌功能不足" } ]
严重时可出现脂肪泻,患儿粪便量显著增多,粪酸臭或恶臭。
[ { "indices": [ 6, 7, 8 ], "label": "sym", "score": 1, "text": "脂肪泻" }, { "indices": [ 12, 13, 14, 15, 16, 17, 18 ], "label": "sym", "score": 1, "text": "粪便量显著增多" }, { "indices": [ 12, 13, 14 ], "label": "ite", "score": 1, "text": "粪便量" }, { "indices": [ 20, 21, 22, 23, 24, 25 ], "label": "sym", "score": 1, "text": "粪酸臭或恶臭" }, { "indices": [ 20 ], "label": "bod", "score": 1, "text": "粪" } ]
遗传性胰腺炎起病年龄多见于5~10岁。
[ { "indices": [ 0, 1, 2, 3, 4, 5 ], "label": "dis", "score": 1, "text": "遗传性胰腺炎" } ]
患者有严重的腹痛伴恶心、呕吐。
[ { "indices": [ 3, 4, 5, 6, 7 ], "label": "sym", "score": 1, "text": "严重的腹痛" }, { "indices": [ 6 ], "label": "bod", "score": 1, "text": "腹" }, { "indices": [ 9, 10 ], "label": "sym", "score": 1, "text": "恶心" }, { "indices": [ 12, 13 ], "label": "sym", "score": 1, "text": "呕吐" } ]
生殖系统异常在男性表现为尿道背侧壁缺如。
[ { "indices": [ 0, 1, 2, 3, 4, 5 ], "label": "dis", "score": 1, "text": "生殖系统异常" }, { "indices": [ 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18 ], "label": "sym", "score": 1, "text": "男性表现为尿道背侧壁缺如" }, { "indices": [ 12, 13, 14, 15, 16 ], "label": "bod", "score": 1, "text": "尿道背侧壁" } ]
肛门直肠异常表现为会阴短、平,肛门前移紧靠尿生殖膈,可伴有肛门狭窄、直肠会阴瘘或直肠阴道瘘。
[ { "indices": [ 0, 1, 2, 3, 4, 5 ], "label": "dis", "score": 1, "text": "肛门直肠异常" }, { "indices": [ 9, 10, 11, 12, 13 ], "label": "sym", "score": 1, "text": "会阴短、平" }, { "indices": [ 9, 10 ], "label": "bod", "score": 1, "text": "会阴" }, { "indices": [ 15, 16, 17, 18, 19, 20, 21, 22, 23, 24 ], "label": "sym", "score": 1, "text": "肛门前移紧靠尿生殖膈" }, { "indices": [ 15, 16 ], "label": "bod", "score": 1, "text": "肛门" }, { "indices": [ 21, 22, 23, 24 ], "label": "bod", "score": 1, "text": "尿生殖膈" }, { "indices": [ 29, 30, 31, 32 ], "label": "dis", "score": 1, "text": "肛门狭窄" }, { "indices": [ 34, 35, 36, 37, 38 ], "label": "dis", "score": 1, "text": "直肠会阴瘘" }, { "indices": [ 40, 41, 42, 43, 44 ], "label": "dis", "score": 1, "text": "直肠阴道瘘" } ]
双侧髂骨截骨可使耻骨联合易于对合,可减小闭合腹壁缺损的张力;可把尿道放入骨盆环内,达到解剖复位;可使尿生殖膈及肛提肌靠拢,协助排尿控制。
[ { "indices": [ 0, 1, 2, 3, 4, 5 ], "label": "pro", "score": 1, "text": "双侧髂骨截骨" }, { "indices": [ 8, 9 ], "label": "bod", "score": 1, "text": "耻骨" }, { "indices": [ 20, 21, 22, 23, 24, 25 ], "label": "dis", "score": 1, "text": "闭合腹壁缺损" }, { "indices": [ 32, 33 ], "label": "bod", "score": 1, "text": "尿道" }, { "indices": [ 36, 37, 38, 39 ], "label": "bod", "score": 1, "text": "骨盆环内" }, { "indices": [ 50, 51, 52, 53 ], "label": "bod", "score": 1, "text": "尿生殖膈" }, { "indices": [ 55, 56, 57 ], "label": "bod", "score": 1, "text": "肛提肌" }, { "indices": [ 64 ], "label": "bod", "score": 1, "text": "尿" } ]
Reed对本病中的巨型多核细胞作了仔细的描述,并否认了这些细胞来自变异型结核的观点,以后这些畸形巨型细胞被命名为Reed-Sternberg细胞(R-S细胞)。
[ { "indices": [ 9, 10, 11, 12, 13, 14 ], "label": "bod", "score": 1, "text": "巨型多核细胞" }, { "indices": [ 29, 30 ], "label": "bod", "score": 1, "text": "细胞" }, { "indices": [ 33, 34, 35, 36, 37 ], "label": "dis", "score": 1, "text": "变异型结核" }, { "indices": [ 46, 47, 48, 49, 50, 51 ], "label": "bod", "score": 1, "text": "畸形巨型细胞" }, { "indices": [ 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71 ], "label": "bod", "score": 1, "text": "Reed-Sternberg细胞" }, { "indices": [ 73, 74, 75, 76, 77 ], "label": "bod", "score": 1, "text": "R-S细胞" } ]
流行病学调查提示疱疹病毒6、巨细胞包涵体病毒、EB病毒感染可能与发病有关。
[ { "indices": [ 8, 9, 10, 11, 12 ], "label": "mic", "score": 1, "text": "疱疹病毒6" }, { "indices": [ 14, 15, 16, 17, 18, 19, 20, 21 ], "label": "mic", "score": 1, "text": "巨细胞包涵体病毒" }, { "indices": [ 23, 24, 25, 26 ], "label": "mic", "score": 1, "text": "EB病毒" }, { "indices": [ 27, 28 ], "label": "dis", "score": 1, "text": "感染" } ]
原因不明38℃以上发热或周期性发热、6个月内体重减轻10%以上、大量盗汗时应想到本病。
[ { "indices": [ 4, 5, 6, 7, 8, 9, 10 ], "label": "sym", "score": 1, "text": "38℃以上发热" }, { "indices": [ 12, 13, 14, 15, 16 ], "label": "sym", "score": 1, "text": "周期性发热" }, { "indices": [ 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30 ], "label": "sym", "score": 1, "text": "6个月内体重减轻10%以上" }, { "indices": [ 32, 33, 34, 35 ], "label": "sym", "score": 1, "text": "大量盗汗" } ]
约2/3的病人就诊时有不同程度的纵隔浸润,引起咳嗽等气管、支气管受压症状。
[ { "indices": [ 11, 12, 13, 14, 15, 16, 17, 18, 19 ], "label": "sym", "score": 1, "text": "不同程度的纵隔浸润" }, { "indices": [ 16, 17 ], "label": "bod", "score": 1, "text": "纵隔" }, { "indices": [ 23, 24 ], "label": "sym", "score": 1, "text": "咳嗽" }, { "indices": [ 26, 27, 28, 29, 30, 31, 32, 33, 34, 35 ], "label": "sym", "score": 1, "text": "气管、支气管受压症状" }, { "indices": [ 26, 27 ], "label": "bod", "score": 1, "text": "气管" }, { "indices": [ 29, 30, 31 ], "label": "bod", "score": 1, "text": "支气管" } ]
目前,儿科ARDS在儿科危重病房平均病死率为50%~70%,治疗转归与机械通气治疗策略、时间、反应、肺部感染类型、年龄、是否控制多脏器系统功能衰竭、并发症处理效果等因素有关。
[ { "indices": [ 3, 4 ], "label": "dep", "score": 1, "text": "儿科" }, { "indices": [ 5, 6, 7, 8 ], "label": "dis", "score": 1, "text": "ARDS" }, { "indices": [ 10, 11 ], "label": "dep", "score": 1, "text": "儿科" }, { "indices": [ 35, 36, 37, 38, 39, 40 ], "label": "pro", "score": 1, "text": "机械通气治疗" }, { "indices": [ 50, 51, 52, 53 ], "label": "dis", "score": 1, "text": "肺部感染" }, { "indices": [ 64, 65, 66, 67, 68, 69, 70, 71, 72 ], "label": "dis", "score": 1, "text": "多脏器系统功能衰竭" } ]
对肺部的损伤可以是直接或间接的;直接损伤如细菌、真菌、病毒感染、溺水、有害气体或液体吸入、肺栓塞、挫伤等;近年,随ICU和外科救治技术应用,持续气道正压通气、心血管手术导致肺缺血再灌注等成为医源性直接肺损伤的重要原因。
[ { "indices": [ 1, 2, 3, 4, 5 ], "label": "sym", "score": 1, "text": "肺部的损伤" }, { "indices": [ 1, 2 ], "label": "bod", "score": 1, "text": "肺部" }, { "indices": [ 18, 19 ], "label": "dis", "score": 1, "text": "损伤" }, { "indices": [ 21, 22 ], "label": "mic", "score": 1, "text": "细菌" }, { "indices": [ 24, 25 ], "label": "mic", "score": 1, "text": "真菌" }, { "indices": [ 27, 28, 29, 30 ], "label": "dis", "score": 1, "text": "病毒感染" }, { "indices": [ 35, 36, 37, 38, 39, 40, 41, 42, 43 ], "label": "sym", "score": 1, "text": "有害气体或液体吸入" }, { "indices": [ 45, 46, 47 ], "label": "dis", "score": 1, "text": "肺栓塞" }, { "indices": [ 49, 50 ], "label": "dis", "score": 1, "text": "挫伤" }, { "indices": [ 57, 58, 59 ], "label": "dep", "score": 1, "text": "ICU" }, { "indices": [ 61, 62 ], "label": "dep", "score": 1, "text": "外科" }, { "indices": [ 70, 71, 72, 73, 74, 75, 76, 77 ], "label": "pro", "score": 1, "text": "持续气道正压通气" }, { "indices": [ 79, 80, 81, 82, 83 ], "label": "pro", "score": 1, "text": "心血管手术" }, { "indices": [ 86, 87, 88 ], "label": "dis", "score": 1, "text": "肺缺血" }, { "indices": [ 90, 91 ], "label": "pro", "score": 1, "text": "灌注" }, { "indices": [ 95, 96, 97, 98, 99, 100, 101, 102 ], "label": "dis", "score": 1, "text": "医源性直接肺损伤" } ]
ARDS/ALI的主要临床表现为急性呼吸困难、呼吸急促、严重低氧血症、胸片异常和肺静态顺应性降低。
[ { "indices": [ 0, 1, 2, 3 ], "label": "dis", "score": 1, "text": "ARDS" }, { "indices": [ 5, 6, 7 ], "label": "dis", "score": 1, "text": "ALI" }, { "indices": [ 16, 17, 18, 19, 20, 21 ], "label": "sym", "score": 1, "text": "急性呼吸困难" }, { "indices": [ 23, 24, 25, 26 ], "label": "sym", "score": 1, "text": "呼吸急促" }, { "indices": [ 28, 29, 30, 31, 32, 33 ], "label": "dis", "score": 1, "text": "严重低氧血症" }, { "indices": [ 35, 36 ], "label": "pro", "score": 1, "text": "胸片" }, { "indices": [ 35, 36, 37, 38 ], "label": "sym", "score": 1, "text": "胸片异常" }, { "indices": [ 40 ], "label": "bod", "score": 1, "text": "肺" }, { "indices": [ 40, 41, 42, 43, 44, 45, 46, 47 ], "label": "sym", "score": 1, "text": "肺静态顺应性降低" } ]
随后呼吸窘迫,吸气费力、发绀,常伴烦躁、焦虑不安,两肺广泛间质浸润,可伴奇静脉扩张、胸膜反应或有少量积液。
[ { "indices": [ 2, 3, 4, 5 ], "label": "sym", "score": 1, "text": "呼吸窘迫" }, { "indices": [ 7, 8, 9, 10 ], "label": "sym", "score": 1, "text": "吸气费力" }, { "indices": [ 12, 13 ], "label": "sym", "score": 1, "text": "发绀" }, { "indices": [ 17, 18 ], "label": "sym", "score": 1, "text": "烦躁" }, { "indices": [ 20, 21, 22, 23 ], "label": "sym", "score": 1, "text": "焦虑不安" }, { "indices": [ 25, 26, 27, 28, 29, 30, 31, 32 ], "label": "sym", "score": 1, "text": "两肺广泛间质浸润" }, { "indices": [ 25, 26 ], "label": "bod", "score": 1, "text": "两肺" }, { "indices": [ 36, 37, 38 ], "label": "bod", "score": 1, "text": "奇静脉" }, { "indices": [ 36, 37, 38, 39, 40 ], "label": "sym", "score": 1, "text": "奇静脉扩张" }, { "indices": [ 42, 43 ], "label": "bod", "score": 1, "text": "胸膜" }, { "indices": [ 42, 43, 44, 45 ], "label": "sym", "score": 1, "text": "胸膜反应" }, { "indices": [ 48, 49, 50, 51 ], "label": "sym", "score": 1, "text": "少量积液" } ]
后期呼吸窘迫和发绀加重,肺部浸润可发展成“白肺”,二氧化碳潴留,心脏停搏或多器官衰竭。
[ { "indices": [ 2, 3, 4, 5 ], "label": "sym", "score": 1, "text": "呼吸窘迫" }, { "indices": [ 7, 8, 9, 10 ], "label": "sym", "score": 1, "text": "发绀加重" }, { "indices": [ 12, 13 ], "label": "bod", "score": 1, "text": "肺部" }, { "indices": [ 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23 ], "label": "sym", "score": 1, "text": "肺部浸润可发展成“白肺”" }, { "indices": [ 22 ], "label": "bod", "score": 1, "text": "肺" }, { "indices": [ 25, 26, 27, 28 ], "label": "bod", "score": 1, "text": "二氧化碳" }, { "indices": [ 25, 26, 27, 28, 29, 30 ], "label": "sym", "score": 1, "text": "二氧化碳潴留" }, { "indices": [ 32, 33 ], "label": "bod", "score": 1, "text": "心脏" }, { "indices": [ 32, 33, 34, 35 ], "label": "sym", "score": 1, "text": "心脏停搏" }, { "indices": [ 37, 38, 39, 40, 41 ], "label": "dis", "score": 1, "text": "多器官衰竭" } ]
1995年,全国危重急救学学术会议(庐山)提出我国ARDS分期诊断标准。
[ { "indices": [ 25, 26, 27, 28 ], "label": "dis", "score": 1, "text": "ARDS" } ]
有些患儿可有不典型表现,如单侧肺弥漫性渗出或肺部影像学特征轻、临床低氧血症和感染症状明显,也可以考虑早期ARDS。
[ { "indices": [ 13, 14, 15, 16, 17, 18, 19, 20 ], "label": "sym", "score": 1, "text": "单侧肺弥漫性渗出" }, { "indices": [ 13, 14, 15 ], "label": "bod", "score": 1, "text": "单侧肺" }, { "indices": [ 22, 23 ], "label": "bod", "score": 1, "text": "肺部" }, { "indices": [ 22, 23, 24, 25, 26, 27, 28, 29 ], "label": "sym", "score": 1, "text": "肺部影像学特征轻" }, { "indices": [ 33, 34, 35, 36 ], "label": "dis", "score": 1, "text": "低氧血症" }, { "indices": [ 38, 39 ], "label": "dis", "score": 1, "text": "感染" }, { "indices": [ 52, 53, 54, 55 ], "label": "dis", "score": 1, "text": "ARDS" } ]
有条件时可以采用CT扫描,尤其对于发现肺泡萎陷、实变有帮助。
[ { "indices": [ 8, 9, 10, 11 ], "label": "pro", "score": 1, "text": "CT扫描" }, { "indices": [ 19, 20 ], "label": "bod", "score": 1, "text": "肺泡" }, { "indices": [ 19, 20, 21, 22, 23, 24, 25 ], "label": "sym", "score": 1, "text": "肺泡萎陷、实变" } ]
在ARDS的早期,首先应考虑通过面罩或气道插管,提供持续气道正压通气(CPAP)或压力支持通气(PSV)或容量支持通气(VSV)。
[ { "indices": [ 1, 2, 3, 4 ], "label": "dis", "score": 1, "text": "ARDS" }, { "indices": [ 16, 17 ], "label": "equ", "score": 1, "text": "面罩" }, { "indices": [ 19, 20, 21, 22 ], "label": "pro", "score": 1, "text": "气道插管" }, { "indices": [ 26, 27, 28, 29, 30, 31, 32, 33 ], "label": "pro", "score": 1, "text": "持续气道正压通气" }, { "indices": [ 35, 36, 37, 38 ], "label": "pro", "score": 1, "text": "CPAP" }, { "indices": [ 41, 42, 43, 44, 45, 46 ], "label": "pro", "score": 1, "text": "压力支持通气" }, { "indices": [ 48, 49, 50 ], "label": "pro", "score": 1, "text": "PSV" }, { "indices": [ 53, 54, 55, 56, 57, 58 ], "label": "pro", "score": 1, "text": "容量支持通气" }, { "indices": [ 60, 61, 62 ], "label": "pro", "score": 1, "text": "VSV" } ]
一般预计病情能够短期缓解的早期ALI/ARDS患者可考虑应用,合并免疫功能低下的ALI/ARDS患儿者早期可首先试用。
[ { "indices": [ 15, 16, 17 ], "label": "dis", "score": 1, "text": "ALI" }, { "indices": [ 19, 20, 21, 22 ], "label": "dis", "score": 1, "text": "ARDS" }, { "indices": [ 33, 34, 35, 36, 37, 38 ], "label": "sym", "score": 1, "text": "免疫功能低下" }, { "indices": [ 40, 41, 42 ], "label": "dis", "score": 1, "text": "ALI" }, { "indices": [ 44, 45, 46, 47 ], "label": "dis", "score": 1, "text": "ARDS" } ]
某些气质特征与频发的梦魇有关。
[ { "indices": [ 7, 8, 9, 10, 11 ], "label": "sym", "score": 1, "text": "频发的梦魇" } ]
相当大部分(20%~40%)的患者有精神分裂样人格、临界人格障碍、精神分裂样人格障碍或精神分裂症。
[ { "indices": [ 18, 19, 20, 21, 22, 23, 24 ], "label": "dis", "score": 1, "text": "精神分裂样人格" }, { "indices": [ 26, 27, 28, 29, 30, 31 ], "label": "dis", "score": 1, "text": "临界人格障碍" }, { "indices": [ 33, 34, 35, 36, 37, 38, 39, 40, 41 ], "label": "dis", "score": 1, "text": "精神分裂样人格障碍" }, { "indices": [ 43, 44, 45, 46, 47 ], "label": "dis", "score": 1, "text": "精神分裂症" } ]
频繁发作的梦魇患者容易得精神病。
[ { "indices": [ 0, 1, 2, 3, 4, 5, 6 ], "label": "sym", "score": 1, "text": "频繁发作的梦魇" }, { "indices": [ 12, 13, 14 ], "label": "dis", "score": 1, "text": "精神病" } ]
各种的紧张、特异的创伤均使梦魇的频率和严重度增加。
[ { "indices": [ 0, 1, 2, 3, 4, 5, 6, 7, 8, 9, 10 ], "label": "sym", "score": 1, "text": "各种的紧张、特异的创伤" }, { "indices": [ 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23 ], "label": "sym", "score": 1, "text": "梦魇的频率和严重度增加" } ]
某些药物,包括L-多巴及类似的药物、β肾上腺素能阻滞剂、REM睡眠抑制药物的撤退可导致或增加梦魇的发生。
[ { "indices": [ 7, 8, 9, 10 ], "label": "dru", "score": 1, "text": "L-多巴" }, { "indices": [ 18, 19, 20, 21, 22, 23, 24, 25, 26 ], "label": "dru", "score": 1, "text": "β肾上腺素能阻滞剂" }, { "indices": [ 28, 29, 30, 31, 32, 33, 34, 35, 36 ], "label": "dru", "score": 1, "text": "REM睡眠抑制药物" }, { "indices": [ 46, 47 ], "label": "dis", "score": 1, "text": "梦魇" } ]
大多数(10%~50%)儿童在3~6岁出现梦魇,其发作是逐渐的。
[ { "indices": [ 21, 22 ], "label": "dis", "score": 1, "text": "梦魇" } ]
对于梦魇发作频繁或较严重者,首先寻找可能影响梦魇发生的一些因素,如各种紧张、焦虑情绪以及某些药物的应用等,有针对性地进行对因治疗。
[ { "indices": [ 2, 3, 4, 5, 6, 7, 8, 9, 10, 11 ], "label": "sym", "score": 1, "text": "梦魇发作频繁或较严重" }, { "indices": [ 22, 23 ], "label": "dis", "score": 1, "text": "梦魇" }, { "indices": [ 35, 36, 37, 38, 39, 40, 41 ], "label": "sym", "score": 1, "text": "紧张、焦虑情绪" } ]
同时对2岁以下的儿童可以在发作的时候用肢体语言安抚,而年长儿则可以尽可能用安抚语言使其平静,并可以在孩子睡眠时为他开一盏灯或将他卧室的门打开,让他充分感觉自己是安全的。
[ { "indices": [ 19, 20 ], "label": "bod", "score": 1, "text": "肢体" } ]
成人以下肢多见。
[ { "indices": [ 3, 4 ], "label": "bod", "score": 1, "text": "下肢" } ]
术后化疗、放疗联合应用。
[ { "indices": [ 2, 3 ], "label": "pro", "score": 1, "text": "化疗" }, { "indices": [ 5, 6 ], "label": "pro", "score": 1, "text": "放疗" } ]
由于肿瘤无症状且生长缓慢,诊断多过晚,预后不良。
[ { "indices": [ 2, 3 ], "label": "dis", "score": 1, "text": "肿瘤" } ]
主要由病毒引起,亦可由A组溶血性链球菌或支原体引起。
[ { "indices": [ 3, 4 ], "label": "mic", "score": 1, "text": "病毒" }, { "indices": [ 11, 12, 13, 14, 15, 16, 17, 18 ], "label": "mic", "score": 1, "text": "A组溶血性链球菌" }, { "indices": [ 20, 21, 22 ], "label": "mic", "score": 1, "text": "支原体" } ]
如患儿同时伴有结膜炎、鼻炎、咳嗽、声音嘶哑或溃疡、皮疹、腹泻等,常提示病毒性咽炎。
[ { "indices": [ 7, 8, 9 ], "label": "dis", "score": 1, "text": "结膜炎" }, { "indices": [ 11, 12 ], "label": "dis", "score": 1, "text": "鼻炎" }, { "indices": [ 14, 15 ], "label": "sym", "score": 1, "text": "咳嗽" }, { "indices": [ 17, 18, 19, 20 ], "label": "sym", "score": 1, "text": "声音嘶哑" }, { "indices": [ 22, 23 ], "label": "dis", "score": 1, "text": "溃疡" }, { "indices": [ 25, 26 ], "label": "sym", "score": 1, "text": "皮疹" }, { "indices": [ 25 ], "label": "bod", "score": 1, "text": "皮" }, { "indices": [ 28, 29 ], "label": "sym", "score": 1, "text": "腹泻" }, { "indices": [ 28 ], "label": "bod", "score": 1, "text": "腹" }, { "indices": [ 35, 36, 37, 38, 39 ], "label": "dis", "score": 1, "text": "病毒性咽炎" } ]
年长儿常以头痛、腹痛、呕吐等非特异性症状起病,伴高热。
[ { "indices": [ 5, 6 ], "label": "sym", "score": 1, "text": "头痛" }, { "indices": [ 5 ], "label": "bod", "score": 1, "text": "头" }, { "indices": [ 8, 9 ], "label": "sym", "score": 1, "text": "腹痛" }, { "indices": [ 8 ], "label": "bod", "score": 1, "text": "腹" }, { "indices": [ 11, 12 ], "label": "sym", "score": 1, "text": "呕吐" }, { "indices": [ 24, 25 ], "label": "sym", "score": 1, "text": "高热" } ]
数小时后出现咽痛。
[ { "indices": [ 6, 7 ], "label": "sym", "score": 1, "text": "咽痛" }, { "indices": [ 6 ], "label": "bod", "score": 1, "text": "咽" } ]
后者轻重不一,重者可造成吞咽困难。
[ { "indices": [ 12, 13, 14, 15 ], "label": "sym", "score": 1, "text": "吞咽困难" } ]
但咽部检查只有三分之一患儿发现典型扁桃体肿大、渗出和咽部充血。
[ { "indices": [ 1, 2, 3, 4 ], "label": "pro", "score": 1, "text": "咽部检查" }, { "indices": [ 17, 18, 19 ], "label": "bod", "score": 1, "text": "扁桃体" }, { "indices": [ 17, 18, 19, 20, 21, 22, 23, 24 ], "label": "sym", "score": 1, "text": "扁桃体肿大、渗出" }, { "indices": [ 26, 27 ], "label": "bod", "score": 1, "text": "咽部" }, { "indices": [ 26, 27, 28, 29 ], "label": "sym", "score": 1, "text": "咽部充血" }, { "indices": [ 29 ], "label": "bod", "score": 1, "text": "血" } ]
年长儿急性咽炎伴发热,但缺乏上呼吸道卡他症状,应首先考虑本病。
[ { "indices": [ 3, 4, 5, 6 ], "label": "dis", "score": 1, "text": "急性咽炎" }, { "indices": [ 8, 9 ], "label": "sym", "score": 1, "text": "发热" }, { "indices": [ 14, 15, 16, 17, 18, 19, 20, 21 ], "label": "sym", "score": 1, "text": "上呼吸道卡他症状" }, { "indices": [ 14, 15, 16, 17 ], "label": "bod", "score": 1, "text": "上呼吸道" } ]
小儿脑积水多为先天性和炎症性病变所致。
[ { "indices": [ 0, 1, 2, 3, 4 ], "label": "dis", "score": 1, "text": "小儿脑积水" }, { "indices": [ 7, 8, 9, 10, 11, 12, 13, 14, 15 ], "label": "dis", "score": 1, "text": "先天性和炎症性病变" } ]
脑室系统与蛛网膜下腔畅通,由于脑脊液的分泌亢进或吸收障碍引起的脑积水称为交通性脑积水。
[ { "indices": [ 0, 1, 2, 3 ], "label": "bod", "score": 1, "text": "脑室系统" }, { "indices": [ 5, 6, 7, 8, 9 ], "label": "bod", "score": 1, "text": "蛛网膜下腔" }, { "indices": [ 15, 16, 17 ], "label": "bod", "score": 1, "text": "脑脊液" }, { "indices": [ 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27 ], "label": "sym", "score": 1, "text": "脑脊液的分泌亢进或吸收障碍" }, { "indices": [ 24, 25, 26, 27 ], "label": "dis", "score": 1, "text": "吸收障碍" }, { "indices": [ 31, 32, 33 ], "label": "dis", "score": 1, "text": "脑积水" }, { "indices": [ 36, 37, 38, 39, 40, 41 ], "label": "dis", "score": 1, "text": "交通性脑积水" } ]
脑积水还有其他很多分类方法,目前尚不统一。
[ { "indices": [ 0, 1, 2 ], "label": "dis", "score": 1, "text": "脑积水" } ]
按照致病原因,可分为先天性和后天性脑积水;按照发病的速度,可分为急性和慢性脑积水;按照颅内压的增高与否,分为高压力性脑积水和正常压力性脑积水;按照发生的部位不同,分为内脑积水和外脑积水。
[ { "indices": [ 10, 11, 12, 13, 14, 15, 16, 17, 18, 19 ], "label": "dis", "score": 1, "text": "先天性和后天性脑积水" }, { "indices": [ 32, 33, 34, 35, 36, 37, 38, 39 ], "label": "dis", "score": 1, "text": "急性和慢性脑积水" }, { "indices": [ 43, 44, 45 ], "label": "ite", "score": 1, "text": "颅内压" }, { "indices": [ 54, 55, 56, 57, 58, 59, 60 ], "label": "dis", "score": 1, "text": "高压力性脑积水" }, { "indices": [ 62, 63, 64, 65, 66, 67, 68, 69 ], "label": "dis", "score": 1, "text": "正常压力性脑积水" }, { "indices": [ 83, 84, 85, 86 ], "label": "dis", "score": 1, "text": "内脑积水" }, { "indices": [ 88, 89, 90, 91 ], "label": "dis", "score": 1, "text": "外脑积水" } ]
4)Arnold-Chiari畸形:因小脑扁桃体、延髓及第四脑室疝入椎管内,使脑脊液循环受阻引起脑积水,常并发脊柱裂及脊膜膨出。
[ { "indices": [ 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16 ], "label": "dis", "score": 1, "text": "Arnold-Chiari畸形" }, { "indices": [ 19, 20, 21, 22, 23 ], "label": "sym", "score": 1, "text": "小脑扁桃体" }, { "indices": [ 19, 20 ], "label": "bod", "score": 1, "text": "小脑" }, { "indices": [ 25, 26 ], "label": "sym", "score": 1, "text": "延髓" }, { "indices": [ 28, 29, 30, 31, 32, 33, 34, 35, 36 ], "label": "sym", "score": 1, "text": "第四脑室疝入椎管内" }, { "indices": [ 28, 29, 30, 31 ], "label": "bod", "score": 1, "text": "第四脑室" }, { "indices": [ 34, 35, 36 ], "label": "bod", "score": 1, "text": "椎管内" }, { "indices": [ 39, 40, 41, 42, 43, 44, 45 ], "label": "sym", "score": 1, "text": "脑脊液循环受阻" }, { "indices": [ 39, 40, 41 ], "label": "bod", "score": 1, "text": "脑脊液" }, { "indices": [ 48, 49, 50 ], "label": "dis", "score": 1, "text": "脑积水" }, { "indices": [ 55, 56, 57 ], "label": "dis", "score": 1, "text": "脊柱裂" }, { "indices": [ 59, 60, 61, 62 ], "label": "dis", "score": 1, "text": "脊膜膨出" } ]
5)扁平颅底:常合并Arnold-Chiari畸形,阻塞第四脑室出口或环池,引起脑积水。
[ { "indices": [ 2, 3, 4, 5 ], "label": "dis", "score": 1, "text": "扁平颅底" }, { "indices": [ 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24 ], "label": "dis", "score": 1, "text": "Arnold-Chiari畸形" }, { "indices": [ 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36 ], "label": "sym", "score": 1, "text": "阻塞第四脑室出口或环池" }, { "indices": [ 28, 29, 30, 31 ], "label": "bod", "score": 1, "text": "第四脑室" }, { "indices": [ 40, 41, 42 ], "label": "dis", "score": 1, "text": "脑积水" } ]
在囟门和颅缝已闭合的较大儿童脑积水,常表现为颅内压增高症(头痛、呕吐和视乳头水肿)。
[ { "indices": [ 1, 2 ], "label": "bod", "score": 1, "text": "囟门" }, { "indices": [ 1, 2, 3, 4, 5, 6, 7, 8 ], "label": "sym", "score": 1, "text": "囟门和颅缝已闭合" }, { "indices": [ 4, 5 ], "label": "bod", "score": 1, "text": "颅缝" }, { "indices": [ 14, 15, 16 ], "label": "dis", "score": 1, "text": "脑积水" }, { "indices": [ 22, 23, 24, 25, 26, 27 ], "label": "dis", "score": 1, "text": "颅内压增高症" }, { "indices": [ 29, 30 ], "label": "sym", "score": 1, "text": "头痛" }, { "indices": [ 29 ], "label": "bod", "score": 1, "text": "头" }, { "indices": [ 32, 33 ], "label": "sym", "score": 1, "text": "呕吐" }, { "indices": [ 35, 36, 37, 38, 39 ], "label": "dis", "score": 1, "text": "视乳头水肿" } ]
部分病人有抽搐发作。
[ { "indices": [ 5, 6, 7, 8 ], "label": "sym", "score": 1, "text": "抽搐发作" } ]
如第三脑室前部和下视丘、漏斗部受累,可出现各种内分泌功能紊乱,例如青春早熟或落后和生长矮小等。
[ { "indices": [ 1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16 ], "label": "dis", "score": 1, "text": "第三脑室前部和下视丘、漏斗部受累" }, { "indices": [ 23, 24, 25, 26, 27, 28, 29 ], "label": "dis", "score": 1, "text": "内分泌功能紊乱" }, { "indices": [ 33, 34, 35, 36, 37, 38, 39 ], "label": "sym", "score": 1, "text": "青春早熟或落后" }, { "indices": [ 41, 42, 43, 44 ], "label": "sym", "score": 1, "text": "生长矮小" } ]
原发症征候,如松果体肿瘤的上视不能,小脑蚓部肿瘤的共济失调等。
[ { "indices": [ 7, 8, 9, 10, 11 ], "label": "dis", "score": 1, "text": "松果体肿瘤" }, { "indices": [ 13, 14, 15, 16 ], "label": "sym", "score": 1, "text": "上视不能" }, { "indices": [ 18, 19, 20, 21, 22, 23 ], "label": "dis", "score": 1, "text": "小脑蚓部肿瘤" }, { "indices": [ 25, 26, 27, 28 ], "label": "dis", "score": 1, "text": "共济失调" } ]
对于早期、发展缓慢或不适于手术治疗的脑积水患儿,则以药物治疗为主,可酌情选用脱水或利尿药。
[ { "indices": [ 13, 14 ], "label": "pro", "score": 1, "text": "手术" }, { "indices": [ 18, 19, 20 ], "label": "dis", "score": 1, "text": "脑积水" }, { "indices": [ 26, 27, 28, 29 ], "label": "pro", "score": 1, "text": "药物治疗" }, { "indices": [ 38, 39, 40, 41, 42, 43 ], "label": "dru", "score": 1, "text": "脱水或利尿药" } ]